An Essential Role for BLNK in Human B Cell Development-Reference-Cited by-同舟云学术

An Essential Role for BLNK in Human B Cell Development

Published:1999-12-03 Issue:5446 Volume:286 Page:1954-1957
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

Minegishi Yoshiyuki¹,Rohrer Jurg¹,Coustan-Smith Elaine²,Lederman Howard M.³,Pappu Rajita⁴,Campana Dario²⁵,Chan Andrew C.⁴,Conley Mary Ellen¹⁵

Affiliation:

1. Department of Immunology,

2. Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.

3. Division of Pediatric Allergy and Immunology, Department of Pediatrics, Johns Hopkins Hospital, Baltimore, MD 21287, USA.

4. Center for Immunology, Division of Rheumatology, Departments of Medicine and Pathology, Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110, USA.

5. Department of Pediatrics, University of Tennessee, College of Medicine, Memphis, TN 38105, USA.

Abstract

The signal transduction events that control the progenitor B cell (pro-B cell) to precursor B cell (pre-B cell) transition have not been well delineated. In evaluating patients with absent B cells, a male with a homozygous splice defect in the cytoplasmic adapter protein BLNK (B cell linker protein) was identified. Although this patient had normal numbers of pro-B cells, he had no pre-B cells or mature B cells, indicating that BLNK plays a critical role in orchestrating the pro-B cell to pre-B cell transition. The immune system and overall growth and development were otherwise normal in this patient, suggesting that BLNK function is highly specific.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference39 articles.

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3. Goitsuka R., et al., J. Immunol. 161, 5804 (1998).

4. BLNK Required for Coupling Syk to PLCγ2 and Rac1-JNK in B Cells

5. Conley M. E., Mathias D., Treadaway J., Minegishi Y., Rohrer J., Am. J. Hum. Genet. 62, 1034 (1998).

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